Although TDP-43 cytoplasmic and nuclear inclusions have been recognized as hallmarks of both FTD and ALS for a long time, it is now shown that genetic variants in TDP-43 account for 1% of all ALS cases and a small number of FTD cases (Sreedharan et al., 2008; Keating et al., 2022). The gene discussed is TARDBP; the disease is frontotemporal dementia.